Primary malignant lymphoma of the urinary bladder. A case report

A patient with primary malignant lymphoma of the urinary bladder is presented. Grossly, the bladder showed multiple submucosal masses. Histologically and immunohistochemically, diffuse B-cell lymphoma of the medium-sized cell type was revealed. On the basis of clinicopathological features, the present case resembled previously recorded cases of bladder lymphoma. The pathogenesis of this primary bladder lymphoma was presumably associated with follicular or chronic cystitis. Primary lymphoma of the bladder is a condition that is very rarely included in series of extranodal lymphomas, and there is a curious sex difference in its occurrence rates between Japan and Western countries. Primary lymphoma of the bladder may be considered a lymphoma that originates fro mucosa-associated lymphoid tissue.     

Lymphomas of mucosa-associated lymphoid tissue arising in the urinary bladder

The clinical, histological and immunohistochemical findings in five primary lymphomas of the urinary bladder are reported. One patient had both lymphoma and transitional cell carcinoma of the bladder. All of the lymphomas showed histological features of mucosa-associated lymphoid tissue (MALT) lymphomas with centrocyte-like cells in all cases. One patient with pre-existing cystitis glandularis showed lymphoepithelial lesions. Biopsies from four patients contained reactive germinal centres and, in two of these, there was follicular colonization by tumour cells. In three patients, repeat biopsies, over several years, showing the changes of MALT lymphoma, were diagnosed as cystitis. We suggest that a large proportion of primary lymphomas of the bladder are lymphomas of MALT and that the characteristic morphological and immunohistochemical features of these tumours should be sought in biopsies containing large numbers of lymphoid cells.     

Primary extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue type with malakoplakia in the urinary bladder: a case report

Primary malignant lymphoma of the urinary bladder is a rare disease constituting less than 1% of neoplasms of the urinary bladder. The most prevalent histological subtype is extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue type (MALT lymphoma). It is frequently associated with chronic cystitis and predominantly occurs in females. On the other hand, malakoplakia is thought to be a reactive granulomatous lesion occurring most prevalently in the genitourinary tracts. It is frequently found in females and often associated with bacterial infection in immunosuppressive status. Here we report a rare case of concurrent primary MALT lymphoma and malakoplakia in the urinary bladder in a 78-year-old Japanese female. Presumably, both lymphoma and malakoplakia are considered to be involved in the antecedent cystitis and might contribute to the development of the urinary bladder tumor of the patient, leading to the occlusion of the right ureter with subsequent hydronephrosis.     

Hepatic MALT lymphoma disclosing a nodal extension

We report a case of a 50-year-old man with chronic viral hepatitis B presenting with a primary hepatic lymphoma of mucosa-associated lymphoid tissue, revealed clinically by a pedicular nodal mass. The liver biopsy showed an active chronic hepatitis and a dense portal lymphoid infiltrate with centrocyte-like cells inducing typical biliary lympho-epithelial lesions. The lymph-node biopsy revealed a marginal zone lymphoma pattern. A monoclonal rearrangement of the immunoglobulin heavy chain gene was detected in the lymph node by polymerase chain reaction. This case of primary hepatic mucosa-associated lymphoid tissue lymphoma confirms that the liver also contains mucosa-associated lymphoid tissue, in which low grade lymphoma can arise.     

Primary bilateral mucosa-associated lymphoid tissue lymphoma of the breast with atypical ductal hyperplasia and localized amyloidosis. A case report and review of the literature

Primary non-Hodgkin lymphoma of the breast is a rare disease. Primary mucosa-associated lymphoid tissue lymphoma is even rarer, and bilateral involvement is exceptional. We describe a case of primary bilateral breast mucosa-associated lymphoid tissue lymphoma with bilateral atypical ductal hyperplasia and bilateral localized amyloidosis in a 64-year-old woman with a history of arthritis and systemic lupus erythematosus and its clinical, histologic, and immunohistochemical features. Microscopic examination of the breast lesion showed dense periductal and perilobular small and plasmacytoid lymphocytes with eosinophilic amyloid in the vessels and the stroma. Bilateral single foci of atypical ductal hyperplasia were also noted. Fine needle aspiration showed small and large lymphocytes and plasma cells. Molecular analysis demonstrated a heavy chain immunoglobulin H gene rearrangement. Flow cytometry studies showed an abnormal B-cell population. The combined histologic, paraffin immunohistochemistry, flow cytometry, and molecular results were considered diagnostic for low-grade mucosa-associated lymphoid tissue lymphoma. The patient underwent bilateral local breast radiation without other organ or site involvement.     

黏膜相关淋巴组织结外边缘区B细胞淋巴瘤及其转化细胞的克隆性

目的分析黏膜相关淋巴组织结外边缘区B细胞淋巴瘤（MALT淋巴瘤）中转化与未转化肿瘤细胞间的克隆联系。方法选具有大细胞转化的、可用于激光显微切割的MALT淋巴瘤6例为研究对象,采用EliVision^TM方法检测bcl-10等蛋白表达,逆转录聚合酶链反应（RT-PCR）检测API2-MALT1融合基因,激光显微切割进行目的细胞群分离,PCR检测免疫球蛋白重链（IgH）基因重排并对产物进行测序分析。结果伴有转化的6例中1例胞质和胞核同时表达bcl-10蛋白,2例检测出API21-ALT1融合基因。同一例不同肿瘤细胞群分别显示同样的基因重排序列。例2和例5在N区和D区有两个核苷酸位点的碱基不同。结论伴有转化的6例MALT淋巴瘤转化和未转化肿瘤细胞群均分别来自同一个肿瘤克隆。     

Primary Malignant Lymphoma of the Urinary Bladder A Case Report

A patient with primary malignant lymphoma of the urinary bladder is presented. Grossly, the bladder showed multiple submucosal masses. Histologically and immunohisto-chemically, diffuse B-cell lymphoma of the medium-sized cell type was revealed. On the basis of clinicopathological features, the present case resembled previously recorded cases of bladder lymphoma. The pathogenesis of this primary bladder lymphoma was presumably associated with follicular or chronic cystitis. Primary lymphoma of the bladder is a condition that is very rarely included in series of extranodal lymphomas, and there is a curious sex difference in its occurrence rates between Japan and Western countries. Primary lymphoma of the bladder may be considered a lymphoma that originates from mucosa associated lymphoid tissue. Acta Pathol Jpn 40: 764-769, 1990.     

Amyloid deposition in primary pulmonary marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue

A rare association between primary pulmonary marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), and pulmonary immunoglobulin light chain (AL) amyloidosis is described in a 65-year-old woman suffering from rheumatoid arthritis (RA). All four nodules in the resected upper lobe of the lung had a similar histological appearance. They were composed of small-medium-sized atypical lymphocytes. Centrocyte-like cells had lymphoepithelial lesions. Immunohistochemically, the tumor cells clonally expressed B-cell markers, and demonstrated clonal rearrangement of the immunoglobulin heavy chain gene on polymerase chain reaction. Based on these findings the diagnosis of primary pulmonary MALT lymphoma was made. In addition, uniform eosinophilic material deposition was identified randomly within the tumor. It was Congophilic and exhibited apple-green birefringence on polarizing microscopy, and remained unaffected by potassium permanganate digestion. Deposited material was immunoreactive to lambda light chain. It was concluded that this material was AL amyloid in primary pulmonary MALT lymphoma. Plasma cells with mRNA of lambda chain was found infiltrated along the border of amyloid deposition. Finally, it is speculated that primary pulmonary MALT lymphoma developing in an autoimmune setting, RA in the present case, is associated with overproduction and abnormal clearance of immunoglobulin by the tumor cells, resulting in AL amyloidosis within the tumor.     

Kuttner's tumor of salivary glands resembling marginal zone B-cell lymphoma of the MALT type: a histopathologic and immunohistochemical study of 7 cases

Kuttner's tumor is a benign inflammatory process of the submandibular gland that presents as a hard mass mimicking a malignant neoplasm clinically. The histologic feature varies according to stage of evolution and severity of inflammation. We report here 7 cases of Kuttner's tumor that morphologically resemble primary salivary gland marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT) type. Histologically, the lobular architecture was distorted and the septa showed sclerosis. There was a dense lymphoplasmacytoid infiltration with lymphoid follicle formation accompanied by loss of acini and ducts. In 4 cases, a few salivary gland ducts contained the lymphoid cells within the epithelium. However, a true lymphoepithelial lesion was observed in none of the 7 cases. Immunohistochemical study demonstrated a disrupted follicular dendritic cell network, which is a characteristic finding of follicular colonization of MALT-type lymphoma. In 6 cases, there were a few small foci of lymphocytes somewhat resembling centrocyte-like cells of MALT-type lymphoma. However, immunohistological study demonstrated the mixed nature of the cells resembling centrocyte-like cells. Moreover, the polytypic nature of B lymphocytes was demonstrated by immunohistochemistry and polymerase chain reaction.     

De novo CD5-positive and Richter's syndrome-associated diffuse large B cell lymphomas are genotypically distinct.

Diffuse large B cell lymphomas (DLBLs) represent a heterogeneous collection of aggressive non-Hodgkin's lymphomas that can arise either de novo or as a result of transformation from chronic lymphocytic leukemia, small lymphocytic lymphoma, follicular lymphomas, or lymphomas of mucosa-associated lymphoid tissue. A small percentage of DLBLs express the CD5 antigen. The majority of these cases have evolved from a pre-existing low grade non-Hodgkin's lymphoma (Richter's syndrome). However, we identified and characterized nine CD5-positive DLBLs in which the patients did not have a previous history or concomitant evidence of chronic lymphocytic leukemia, small lymphocytic lymphoma, follicular lymphoma, or mucosa-associated lymphoid tissue-associated non-Hodgkin's lymphoma, suggesting that they arose de novo. All nine cases expressed CD20 and monotypic immunoglobulin, all eight cases examined expressed CD19, CD22 and CD43, eight of the nine cases expressed HLA-DR, and two of eight cases expressed CD11c. None of the cases expressed CD3, CD10, CD11b, CD21, CD23 or CD30. CD5 expression by these cells was found to be identical to that of CD5-positive B cell chronic lymphocytic leukemia by quantitative polymerase chain reaction analysis of CD5 mRNA. These nine de novo CD5-positive DLBLs exhibited clonal immunoglobulin heavy and light chain gene rearrangements but lacked integration of the Epstein-Barr virus genome and structural alterations of the bcl-1, bcl-2, c-myc, H-ras, K-ras, and N-ras proto-oncogenes and the p53 tumor suppressor gene. However, bcl-6 proto-oncogene rearrangement, which is involved in chromosome band 3q27 aberrations, was found in four cases (44.4%). This is comparable with the frequency of bcl-6 gene rearrangement in CD5-negative DLBL. In contrast, bcl-6 gene rearrangement was absent in six cases of DLBL associated with Richter's syndrome. These findings suggest that de novo CD5-positive DLBLs are genotypically similar to CD5-negative DLBLs and may be pathogenetically distinct from the DLBLs associated with Richter's syndrome.     

肺黏膜相关淋巴组织边缘区B细胞淋巴瘤及良性淋巴组织增生性疾病的临床病理分析

目的 研究肺原发性黏膜相关淋巴组织边缘区B细胞(MALT)淋巴瘤及良性淋巴组织增生性疾病的临床病理形态、免疫组织化学表型和B细胞重链基因重排,比较肺MALT淋巴瘤和良性淋巴组织增生性疾病的差异.方法 回顾性的分析原发性肺MALT淋巴瘤13例,7例肺良性淋巴组织增生性疾病资料.对标本行常规HE染色,EnVision免疫组织化学染色(抗体包括AE1/AE3、CD20、CD79α、CD3、CD5、CD10、CD21、bel-2、bcl-6、cyclinD-1)及免疫球蛋白重链IgH基因重排检测.结果 13例肺MALT淋巴瘤,细胞成分多样,分别由不同比例的小淋巴细胞样细胞、中心细胞样细胞、单核样B细胞组成,常伴有浆细胞分化.肿瘤细胞以弥漫性和滤泡边缘区排列为主,常见反应性淋巴滤泡和滤泡中心的植入.肿瘤细胞呈串珠状直接侵犯肺泡间隔和沿支气管血管束向周边及肺膜扩散.MALT淋巴瘤中,均未见坏死.9例可见肿瘤细胞侵犯血管壁,6例可见胸膜累及,2例肺门淋巴结侵犯.9例肺MALT淋巴瘤可见淋巴上皮样病变,免疫组织化学显示上皮细胞内的淋巴细胞CD20阳性,CD3阴性.7例肺良性淋巴组织增生性疾病,2例可见淋巴上皮样病变,免疫组织化学显示,其淋巴上皮样病变内的淋巴细胞,部分CD20阳性,部分CD3阳性.9例肺MALT淋巴瘤进行了免疫球蛋白重链IgH基因重排,8例阳性;7例良性淋巴组织增生性疾病均为阴性.结论 肺MALT淋巴瘤在细胞组成和排列上与其他部位结外MALT淋巴瘤相同,肿瘤细胞呈串珠状直接侵犯肺泡间隔和沿支气管血管束向周边及肺膜扩散.在肺内淋巴上皮样病变常见于MALT淋巴瘤,并有助于诊断,但并非其特异性病变,一些肺的反应性淋巴组织增生也可出现,用免疫组织化学有助于区别两种病变.免疫球蛋白重链IgH基因重排可以帮助鉴别肺MALT淋巴瘤和良性淋巴组织增生性疾病.     

Primary hepatic lymphoma of mucosa-associated lymphoid tissue type: a case report with cytogenetic study

Primary hepatic lymphoma of mucosa-associated lymphoid tissue type is extremely rare. Only 38 cases have been reported to date. A case of a 59-year-old man with Helicobacter pylori-resistant gastric ulcers and Buerger disease who was followed up since 1999 is reported. A 2-cm hepatic nodule was incidentally found during partial gastrectomy and corresponded to mucosa-associated lymphoid tissue-type lymphoma without underlying liver disease. Molecular studies showed a clonal immunoglobulin heavy-chain gene rearrangement. Investigations for the mucosa-associated lymphoid tissue lymphoma-associated translocations t(11;18) and t(14;18), as well as the t(3;14)(q27;q32), were negative, whereas trisomy 3 and trisomy 18 were detected.     

Primary low-grade MALT lymphoma of the gallbladder

We report a case of primary low-grade B-cell lymphoma of the mucosa-associated lymphoid tissue (low-grade MALT lymphoma) in the gallbladder. A 58-year-old woman suspected of gallbladder carcinoma underwent laparoscopic cholecystectomy. Microscopic examination of the gallbladder demonstrated lymphoid cell infiltration forming lymphoid follicles with hyperplastic secondary follicles. The surrounding monocytoid B cells and centrocyte-like cells selectively infiltrated the crypt epithelium forming lympho-epithelial lesions. Plasma cells were also noted beneath the mucosal epithelium. Bile culture revealed the Gram-negative bacilli Enterococcus faecalis and Morganella morganii. Immunoglobulin heavy chain gene rearrangement was confirmed using polymerase chain reaction (PCR) and oligoclonal lymphoid proliferations was detected. Because autoimmune diseases, or chronic inflammatory disorders, seem to correlate with the occurrence of MALT lymphoma, Gram-negative bacterial infection could also be considered as a prodrome of MALT lymphoma of the gallbladder.     

Malignant lymphoma of the urinary bladder: a clinicopathological study of 11 cases

AIM: To report the clinical and histological features and outcome of primary and secondary malignant lymphomas of the urinary bladder. METHODS: Eleven cases of malignant lymphoma of the urinary bladder were obtained from the registry of cases at St Bartholomews and the Royal London Hospitals. The lymphomas were classified on the basis of their morphology and immunophenotype, and the clinical records were reviewed. RESULTS: There were six primary lymphomas: three extranodal marginal zone lymphomas of mucosa associated lymphoid tissue (MALT) type and three diffuse large B cell lymphomas. Of the five secondary cases, four were diffuse large B cell lymphomas, one secondary to a systemic follicular follicle centre lymphoma, and one nodular sclerosis Hodgkins disease. Four patients with secondary lymphoma for whom follow up was available had died of disease within 13 months of diagnosis. Primary lymphomas followed a more indolent course. In one case, there was evidence of transformation from low grade MALT-type to diffuse large B cell lymphoma. The most common presenting symptom was haematuria. Cystoscopic appearances were of solid, sometimes necrotic tumours resembling transitional cell carcinoma, and in one case the tumours were multiple. These cases represented 0.2% of all bladder neoplasms. CONCLUSIONS: Diffuse large B cell lymphoma and MALT-type lymphoma are the most common primary malignant lymphomas of the bladder. Lymphoepithelial lesions in MALT-type lymphoma involve transitional epithelium, and their presence in high grade lymphoma suggests a primary origin owing to transformation of low grade MALT-type lymphoma. Primary and secondary diffuse large B cell lymphomas of the bladder are histologically similar, but the prognosis of the former is favourable.     

Primary gastric lymphoma—a tumour of mucosa-associated lymphoid tissue. A histological and immunohistochemical study of 36 cases

A series of 36 cases of non-Hodgkin's lymphoma of the stomach have been analysed using routine histological techniques and immunohistochemistry. All cases were categorized as follicle centre cell lymphomas. Apart from two cases who had nodal lymphomas followed by gastric lymphomas, all cases appeared to represent primary lymphoma of mucosa-associated lymphoid tissue. It is proposed that the morphology and behaviour of these tumours reflect their origin from gut-associated lymphoid tissue. Physiologically well-differentiated examples show monotypic plasmacytic differentiation. Infiltration of gastric glands by follicle centre cells forming characteristic lympho-epithelial lesions is, we believe, a pathognomonic feature of primary gastric lymphoma. The spread of these tumours is within the mucosa-associated lymphoid tissues involving, in particular, the nasopharynx and lung but seldom spreading to peripheral lymph nodes or bone marrow. This concept of gastric lymphomas as primary neoplasms of gut-associated lymphoid tissue has important implications with respect to the investigation and treatment of this disease.     

Low-grade B cell lymphoma of mucosa-associated lymphoid tissue in the thymus of a patient with rheumatoid arthritis

The majority of thymlc lymphomas are either lymphoblastic lymphoma, large B cell lymphoma or Hodgkin's disease, and other types of non-Hodgkin lymphoma are rare. A case of low-grade B cell lymphoma of mucosa-associated lymphoid tissue (MALT) In the thymus is reported. A 55-year-old Japanese female with a history of rheumatoid arthritis (RA) complained of back pain. A mediastinal tumor was identified by computerized tomography and magnetic resonance imaging, and the thymus was resected through median sternotomy. The solid and nodular tumor had several small satellite extensions and was completely confined to within the thymus. Hlstologically, monotonous medium-sized centrocyte-like cells occupied the medulla of the thymus and Infiltrated Hassan's corpuscles (lymphoepithellal lesions). Immunohistochemically, tumor cells were positive for CD20 and CD79a. IgA and kappa light chain restriction were also found in plasmacytoid cells in the tumor. Clonal rearrangement of the immunoglobulin heavy chain gene was demonstrated by polymerase chain reaction. This case was diagnosed as MALT-type low-grade B cell lymphoma In the thymus. This is the first report of low-grade B cell lymphoma In the thymus associated with RA. As autoimmune diseases are known to be associated with lymphoid neoplasms, It is suggested that the RA played an Important role in the development of malignant lymphoma in this case.     

SPLENIC MARGINAL ZONE EXPANSION IN B-CELL LYMPHOMAS OF GASTROINTESTINAL MUCOSA-ASSOCIATED LYMPHOID TISSUE (MALT) IS REACTIVE AND DOES NOT REPRESENT HOMING OF NEOPLASTIC LYMPHOCYTES

It has been suggested that lymphocytes of mucosa-associated lymphoid tissue (MALT) arise from marginal zone cells and that MALT-derived lymphomas may spread to other extra-nodal sites by homing to marginal zones in different tissues.¹ Marginal zone expansion has been observed in spleens removed during surgery for gastrointestinal MALT lymphoma, which was sufficiently extreme in some cases to suggest neoplastic involvement. To investigate this phenomenon, polymerase chain reaction (PCR) amplification of immunoglobulin heavy chain gene fragments was performed to demonstrate B-cell clonality in gastrointestinal MALT lymphomas and spleens from 11 patients. Monoclonal PCR products were obtained from 9 of the 11 gastrointestinal tumours but from none of the accompanying spleens. One additional spleen, for which the accompanying gastric lymphoma tissue was unavailable for review, yielded a monoclonal product. However, obvious lymphoma deposits were present in this specimen, in addition to marginal zone enlargement. It is concluded that splenic marginal zone expansion accompanying gastrointestinal MALT lymphoma is correctly interpreted as being reactive. Splenic involvement by MALT lymphoma is uncommon and does not show preferential colonization of the marginal zone to suggest homing of MALT-derived cells to this site.